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Editorial commentaries

What is impact?
Matthew C Kiernan
built up from 1920 is reected through our achievement of the longest citation halflife of any journal across the clinical neurosciences. In other words, the original JNNP manuscripts continue to be heavily cited, many in the thousands,2 3 with some notching up more than 10 000 citations,4 further serving to reinforce the journals standing as a neuroscience trailblazer. How then does a manuscript become highly cited, a critical work from which others model and further develop their theories and practice? To help us understand the process, in this issue of JNNP we launch Impact Commentaries (gure 1), a monthly series which will provide a modern perspective on some of the most highly cited JNNP papers of all time. Where possible, we have approached the authors of the original study. In those instances where the author is no longer alive, we have asked key opinion leaders to comment on the original science and subsequent course of the ndings presented, to decipher the reasons behind the success of each publication. In addition to providing pearls of wisdom, these commentaries provide newcomers, such as neurology trainees, with an opportunity to put the discoveries and developments into an historical context. Unfortunately, it is all too rare to have the opportunity to get the long view from the original author of research that in retrospect has been a blockbuster. With our new monthly Impact Commentaries, we will focus on the opinions that set the scene and then go beyond the research study to discover how it inuenced important developments in the eld, in some cases over many decadesda perspective that newcomer journals are unable to provide.

The word impact is in peril of becoming hackneyed terminology. In modern parlance its meaning appears to have undergone more costume changes than Lady Gaga on a whistle-stop tour! And in the world of medical publishing the denition of impact is nowhere more nebulous than where a ground breaking paper, and a review that merely cites a ground breaking paper, are measured using the same impact criteria. How then does one judge the impact of a publication, both at the time and thereafter? For instance, in high turnover publications such as daily newspapers, initial impact may be phenomenal, although transitory, from front page scoop, to sh and chips wrapping in the space of a single day. When considering medicine, and particularly medical publications, presumably the desired impact would mean that manuscripts were read, information conveyed and subsequently adopted by the reader, and as a consequence, practice was changed with the reasonable hope and anticipation that patient outcomes would improve. When considering the role of JNNP in such a process, in addition to immediate impact, the journals current high standing has been built up over close to a century of publishing the worlds seminal neuroscience publications.1 As proof of longevity and ongoing relevance, akin to opening bottles of wine from an established cellar for the palate of the connoisseur, the success of the journals immense archive
Correspondence to Professor M C Kiernan, Editor-in-Chief of JNNP, Neuroscience Research Australia, Barker Street, Randwick, Sydney, NSW 2031, Australia; m.kiernan@unsw.edu.au J Neurol Neurosurg Psychiatry January 2012 Vol 83 No 1

While looking at impact from the past, JNNP continues to be excited about the future. Our ultimate goal is to identify key new developments and potential future discoveries in the constantly evolving world of neuroscience. The past year has seen JNNP receive more than 3000 submissions, although the higher submission rates inevitably generate greater selectivity. Already, a signicant proportion of these recently published manuscripts, covering the entire realm of clinical neuroscience, are well on the way to becoming citation classics in their own right.5e16 Add to this reviews from experienced clinician researchers, regular podcasts and the recently launched JNNP blog, and a very 21st century notion of impact begins to develop. Borrowing a sentiment from the musician Brian Eno, once youve shocked, you cant shock again with the same tune. This year at JNNP we will be embracing that old rocker s conviction by both examining why the papers that shaped the world of neuroscience did so and seeking out the future classics that will make their impact on the brain and mind sciences. We look forward to an exciting and challenging year ahead.
Competing interests None. Provenance and peer review Commissioned; not externally peer reviewed. Accepted 28 October 2011 J Neurol Neurosurg Psychiatry 2012;83:1e2. doi:10.1136/jnnp-2011-301738

REFERENCES
1. 2. Kiernan MC. The realm of neurologydpast, present and future. J Neurol Neurosurg Psychiatry 2011;82:1. Scoville WB, Milner B. Loss of recent memory after bilateral hippocampal lesions. J Neurol Neurosurg Psychiatry 1957;20:11e21. Hughes AJ, Daniel SE, Kilford L, et al. Accuracy of clinical-diagnosis of idiopathic Parkinsons-diseaseda clinicopathological study of 100 cases. J Neurol Neurosurg Psychiatry 1992;55:181e4. Hamilton M. A rating scale for depression. J Neurol Neurosurg Psychiatry 1960;23:56e62. Buse DC, Manack A, Serrano D, et al. Sociodemographic and comorbidity proles of chronic migraine and episodic migraine sufferers. J Neurol Neurosurg Psychiatry 2010;81:428e32. Jankovic J, Jimenez-Shahed J, Brown LW. A randomised, double-blind, placebo-controlled study of topiramate in the treatment of Tourette syndrome. J Neurol Neurosurg Psychiatry 2010;81:70e3. Fleuren JF, Voerman GE, Erren-Wolters CV, et al. Stop using the Ashworth Scale for the assessment of spasticity. J Neurol Neurosurg Psychiatry 2010;81:46e52. Fois AF, Wotton CJ, Yeates D, et al. Cancer in patients with motor neuron disease, multiple sclerosis and Parkinsons disease: record linkage studies. J Neurol Neurosurg Psychiatry 2010;81:215e21.

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Figure 1 Impact Commentaries, launched in this months issue, provide a modern perspective on the most highly cited JNNP papers of all time.

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Editorial commentaries

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Logroscino G, Traynor BJ, Hardiman O, et al. Incidence of amyotrophic lateral sclerosis in Europe. J Neurol Neurosurg Psychiatry 2010;81:385e90. Topakian R, Barrick TR, Howe FA, et al. Blood-brain barrier permeability is increased in normal-appearing white matter in patients with lacunar stroke and leucoaraiosis. J Neurol Neurosurg Psychiatry 2010;81:192e7. Ibarretxe-Bilbao N, Ramirez-Ruiz B, Junque C, et al. Differential progression of brain atrophy in Parkinsons

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disease with and without visual hallucinations. J Neurol Neurosurg Psychiatry 2010;81:650e7. Uncini A, Manzoli C, Notturno F, et al. Pitfalls in electrodiagnosis of Guillain-Barre syndrome subtypes. J Neurol Neurosurg Psychiatry 2010;81:1157e63. Douglass CP, Kandler RH, Shaw PJ, et al. An evaluation of neurophysiological criteria used in the diagnosis of motor neuron disease. J Neurol Neurosurg Psychiatry 2010;81:646e9. Zampieri C, Salarian A, Carlson-Kuhta P, et al. The instrumented timed up and go test: potential

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outcome measure for disease modifying therapies in Parkinsons disease. J Neurol Neurosurg Psychiatry 2010;81:171e6. Kagi G, Bhatia KP, Tolosa E. The role of DAT-SPECT in movement disorders. J Neurol Neurosurg Psychiatry 2010;81:5e12. Charlton RA, Schiavone F, Barrick TR, et al. Diffusion tensor imaging detects age related white matter change over a 2 year follow-up which is associated with working memory decline. J Neurol Neurosurg Psychiatry 2010;81:13e19.

The expanding phenotype of CLIPPERS: is it a disease or a syndrome?

Jun-ichi Kira
Chronic lymphocytic inammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a newly named pontine-centric inammatory disorder.1 The cardinal feature of the disease is punctate gadolinium enhancement peppering the pons on MRI. The unique MRI features of this disorder have attracted many neurologists attention leading to the publication of several case reports recently.2e5 The biopsied pontine pathology from the original study revealed a marked perivascular and parenchymal CD3-postive T-cell inammation without any specic pathology.1 However, because of the lack of a specic biomarker and long-term follow-up, the nosological position of CLIPPERS is still to be established. The paper by Simon and colleagues6 (see page 15) reports ve additional cases of CLIPPERS with detailed pathology and long-term evaluation, expanding the clinical, neuroimaging and pathological phenotype of this disorder: (1) cognitive impairment was seen in four of ve cases along with cerebral atrophy in three of them; (2) MRI lesions were distributed not only in the pons but also in the brachium ponti and cerebellum, which later culminated in severe atrophy of the cerebellum and brachium ponti; (3) prominent CD4-positive T lymphocytic as well as histiocytic inltrates were observed, involving both small arteries and veins but with few B cells. Neuroaxonal injury was also found but there was no evidence of vasculitis (destruction of the vessel wall with brinoid necrosis).6 Based on the distribution of MRI lesions, Simon and colleagues propose an amendment of the disorder to chronic lymphocytic inammation with pontocerebellar perivascular enhancement responsive to steroids (CLIPPERS).6 Lesions may occur in the spinal cord, basal ganglia or cerebral white matter. The perivascular gadolinium enhancement pattern and steroid-responsiveness indicate the autoimmune/inammatory nature of this condition. These authors6 and others1 carried out extensive laboratory and pathological surveys to exclude specic causes for the condition, such as sarcoidosis, histiocytosis, lymphoma, granulomatosis, multiple sclerosis, isolated angiitis of the central nervous system, Lyme disease, Whipple disease, Bickerstaff brainstem encephalitis, Behcets disease and Sjgrens syndrome, suggesting that CLIPPERS is an independent disease entity. However, there appears to be some overlap with other autoimmune/inammatory brainstem-predominant encephalitis, especially brainstem type of neuro-Behcets disease and Sjgrens syndrome. Pittock and colleagues1 found no evidence of systemic illness; however,

Department of Neurology, Neurological Institute, Graduate School of Medical Sciences, Kyushu University, Japan Correspondence to Professor Jun-ichi Kira, Department of Neurology, Neurological Institute, Graduate School of Medical Sciences, Kyushu University, Fukuoka 812-8582, Japan; kira@neuro.med.kyushu-u.ac.jp 2

Simon and colleagues6 reported additional subclinical systemic ndings in some cases, namely antinuclear antibody SS-A, lymphocytic conjunctival inltrate, lymphocytic sialadenitis and parotid uptake on gallium scan. Neuro-Behcets disease is well known and frequently affects the pons and cerebellum. This disease occasionally presents without apparent mucocutaneo-ocular manifestations,7 8 showing progressive cerebellar ataxia and prominent pontine and cerebellar atrophy. Such patients can also benet from early steroid therapy. Cognitive impairment, rst described by Simon and colleagues6 in CLIPPERS, is also frequently encountered in Behcets disease. On MRI, enhancement of lesions in the pons and middle cerebellar peduncles frequently shows a mottled nonconuent pattern similar to that of CLIPPERS.9e11 At the chronic stage, severe atrophy of the basis pontis and cerebellum is common. Pathologically, Behcets disease shows perivascular inltration of T cells and macrophages/monocytes with few B cells, mainly involving venules but also occasionally small arteries.12 Examinations of needle reaction, HLA-DR51 and interleukin 6 in the cerebrospinal uid are essential to differentiate brainstem type of neuro-Behcets disease from CLIPPERS. So far, all cases with CLIPPERS have been reported from Western countries. Behcets disease is prevalent in Mediterranean countries, the Middle East and Japan. It is interesting to know whether there is any racial preponderance for this condition. Cerebellar and brainstem involvement has also been repeatedly reported in Sjgrens syndrome,13e15 while sicca symptoms may not be clinically overt. MRI features of brainstem involvement in primary Sjgrens syndrome occasionally presents punctate gadolinium-enhancing foci peppering the pons, middle cerebellar peduncles, cerebellar hemispheres and vermis, and mesencephalon, which are quite similar to those of CLIPPERS.15 The subclinical involvement of exocrine glands found in some CLIPPERS cases6 suggests
J Neurol Neurosurg Psychiatry January 2012 Vol 83 No 1

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What is impact?
Matthew C Kiernan J Neurol Neurosurg Psychiatry 2012 83: 1-2

doi: 10.1136/jnnp-2011-301738

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